Polysplenia syndrome

Case contributed by Dr Abdallah Al Khateeb

Presentation

Imaging work-up for new diagnosis of breast cancer (staging).

Patient Data

Age: 45 years
Gender: Female
CT

Multiple splenules of variable size in the left upper quadrant.

Left-sided inferior vena cava with hemiazygous continuation. Normal caliber and course of the azygous vein. Direct drainage of the hepatic veins into the right atrium.

Bi-lobed lungs; no right horizontal fissure.

Other findings: cholelithiasis, bariatric surgery, and paraesophageal hiatal hernia.

Case Discussion

Altogether, the findings are best explained by polysplenia syndrome. The presentation of this entity is variable, and is related to the associated pathologies. In this patient, the pathology was incidentally found.

Polysplenia should not be mistaken for splenosis. The latter is acquired splenic tissue ectopia that usually follows a traumatic splenic injury or splenectomy. They can easily be differentiated by patient history and the presence of other associated pathologies. 

The most common associated finding in polysplenia syndrome is interruption of the inferior vena cava with azygous or hemiazygous continuation. In this patient, it is with the less common hemiazygous continuation.

Awareness of the other possible associations is sensible.

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